Me ogy dic ol in c e n a O n f d o P Journal of Oncology Medicine & l r a a n c r Doghri, J Oncol Med Pract 2017, 2:2 t u i c o e J Practice Case Report Open Access Teratoid Medulloepithelioma: A Rare Intraocular Tumor of a Child Raoudha Doghri, Lamia Charfi, Yoldez Houcine*, Nadia Boujelbene, Karima Mrad and Maha Driss Department of Pathology, Salah Azaïz Institute, 1006 Bab Saadoun,Tunis, Tunisia *Corresponding author: Yoldez Houcine, Department of Pathology, Salah Azaïez Institute, 1006 Bab Saadoun, Tunis, Tunisia, Tel: 0021671577850; Fax: 0021671574725; E-mail: [email protected] Received date: August 24, 2017; Accepted date: September 18, 2017; Published date: September 25, 2017 Copyright: © 2017 Doghri R, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Medulloepithelioma is a rare congenital neuroepithelial tumor commonly arising from the non-pigmented ciliary body epithelium and rarely from iris, retina or the optic nerve. It occurs in patient under 10 years. It is a rare neuroepithelial tumor and is the second most frequent intraocular tumour in children after retinoblastoma. Unlike cases reported in the literature in which the tumour recurs rapidly, recurrence occurred in our case five years later. Keywords Medulloepithelioma; Childhood; Ocular tumour; Standardized echo graphic examination was normal. Tomographic Differential diagnosis; Histopathology sections of the head and orbits confirmed the presence of a solitary intraocular tumour situated on the posterior chamber (Figure 1). The Introduction patient underwent enucleation of the left eye. The specimen was preserved in 10% formalin and submitted for histological examination. Intraocular medulloepithelioma is a congenital tumor of the ciliary No adjuvant treatment was prescribed to the patient. epithelium that typically presents during the first decade of life. The histologic diagnosis is based on characteristic ribbons of pseudo Macroscopic description stratified neuroepithelium admixed with loose mesenchymal tissue rich in hyaluronic acid, vaguely resembling developing retina and The enucleated eye measured 25 × 25 × 15 mm. The eyeball was vitreous. Malignant medulloepitheliomas consist of a proliferation of completely filled with a whitish and partially necrotic tumour that neuroblasts, which in areas can be indistinguishable from infiltrated the anterior chamber, iris and ciliary body. retinoblastoma. Given the rarity of medulloepithelioma, there is limited information on long-term survival. The aim of this article is to Microscopic description report a case of intraocular medulloepithelioma with extremely rare Sections from paraffin-embedded tissue stained with haematoxylin evolution. and eosin (H&E) revealed in non-necrotic areas a highly cellular tumour (Figure 2). Case Report A 5-year-old girl with a history of a left eye’s congenital glaucoma operated 3 years ago was complained of lack of her vision. Clinical examination found a hypervascularisation of the iris. It also revealed the presence of a nodule in the posterior portion of the globe. Figure 2: Tumour proliferation with increased number of cells and necrotic areas. Figure 1: Well limited intra ocular tumor mass. J Oncol Med Pract, an open access journal Volume 2 • Issue 2 • 1000113 Citation: Doghri R, Charfi L, Houcine Y, Boujelbene N, Mrad K, et al (2017) Teratoid Medulloepithelioma: A Rare Intraocular Tumor of a Child. J Oncol Med Pract 2: 113. Page 2 of 3 The tumour cells were poorly differentiated and sometimes Discussion pigmented, with a scanty basophilic cytoplasm. Nuclear showed significant pleomorphism, mitoses and hyperchromasia. Architecture Medulloepithelioma (ME) is a rare neuroepithelial tumour. It is the was solid, trabecular and pseudo-glandular structures alternating with second most frequent intraocular tumour in children after cystic spaces. Differentiation into Homer-Wright and Flexner- retinoblastoma [1]. Medulloepithelioma arises from the medullary Wintersteiner-like rosettes was present (Figure 3). epithelium of the ciliary body. Occasionally, it occurs in the optic nerve [2,3] and in the retinal stalk [4,5]. It occurs in children (middle age 5 years) without sex predilection [6]. This tumour is characterized by a slow evolution. It becomes symptomatic if it is voluminous. Clinical manifestations are blindness or decrease in visual acuity (39%), painless (30%), leucokoria (18%) and mass (18%) [1]. Related features include secondary glaucoma, iris neovascularization, cataract, lens subluxation, lens coloboma, retrolental neoplastic and cyclitic membrane [6]. Imaging techniques generally demonstrates a cyst or mass involving the ciliary body or iris. The typical echo graphical aspect is the presence of an oval or slit-like minimally reflective area in a low-to-medium reflective irregularly structured mass [7]. Zimmerman has classified medulloepithelioma as teratoid and nonteratoid types [8]. The nonteratoid medulloepithelioma (diktyoma) is a pure proliferation of cells of the medullary epithelium. teratoid medulloepithelioma, which represents 40% of all medulloepithelioma, is distinguished by the additional presence of heterologous elements, particularly cartilage, skeletal muscle, and brain tissue. It arises commonly from the non-pigmented ciliary epithelium, rarely from retina or the optic nerve [9]. Figure 3: Differentiation into Homer-Wright and Flexner-Winter Most intraocular medulloepitheliomas occur sporadically. However, Steiner-like rosettes. cytogenetic abnormalities of DICER1 were reported in one case and association with pleuropulmonary blastoma in 5% of cases [10,11]. In some area, differentiation into calcified brain-like tissue (Figure Histologically, medulloepithelioma contain elements that closely 4) with fibrillary and neuroid focus was noted. The tumour didn’t resemble the medullary epithelium and may contain structures infiltrate the choroids or the sclera. The diagnosis of teratoid resembling those derived from the optic vesicle or optic cup, retinal medulloepithelioma was made. pigmented epithelium, non-pigmented and pigmented ciliary epithelium, and vitreous [7]. In the areas of proliferating medullary epithelium, the tumor cells are characteristically arranged in cords and sheets separated by cystic spaces containing hyaluronic acid. They have considerable pleomorphism and are pleuripotential. Structures of undifferentiated cells resembling those of retinoblastoma (Homer-Wright and Flexner-Wintersteiner-like rosettes) may be noted. However, most rosettes in these tumours have a lumen surrounded by more than a single layer of cells. Small cords of pigmented neuroepithelial cells are often present; they are usually enmeshed in non-pigmented tissue. Malignant medulloepithelioma may not always differ appreciably from the benign tumours. The features of malignancy include the presence of undifferentiated neuroblastic cells, mitotic activity, and the presence of areas resembling soft tissue sarcoma [12]. But the most reliable criteria are invasiveness and extension outside the eye [8]. The most frequently observed heterotopic tissue is hyaline cartilage. Brain-like tissue and skeletal muscle may also be noted. The histologic differential diagnosis of medulloepithelioma is broad, ranging from retinoblastoma and sarcoma to ocular teratoma, ciliary Figure 4: calcified brain-like tissue. epithelial adenoma and adenocarcinoma [6-8]. Medulloepithelioma is the only ocular childhood neoplasm that can histologically mimic retinoblastoma and constitute a diagnostic problem for the pathologist. Five years later, despite a total enucleation, the patient was suffered from an orbital recurrence treated with local radiation therapy. J Oncol Med Pract, an open access journal Volume 2 • Issue 2 • 1000113 Citation: Doghri R, Charfi L, Houcine Y, Boujelbene N, Mrad K, et al (2017) Teratoid Medulloepithelioma: A Rare Intraocular Tumor of a Child. J Oncol Med Pract 2: 113. Page 3 of 3 Enucleation is generally recommended because local resection is References insufficient and the recurrent rate is high. The role of radiotherapy and chemotherapy are unknown. 1. Mojgan D (2014) Medulloepithelioma of the ciliary body. Bulletin of the French Division of the AIP 60: 159-164 The prognosis for medulloepithelioma limited to the eye is good 2. Reese AB (1957) Medulloepithelioma (dictyoma) of the optic nerve. Am J because of the slow growth rate, and rare lymphatic and Ophthalmol 44: 4-6. haematogenous dissemination [7]. 3. Green WR, Iliff WJ, Trotter RR (1974) Malignant teratoid medulloepithelioma of the optic nerve. Arch Ophthalmol 91: 451. In a series of 41 patients with ciliary body ME, systemic metastasis 4. Anderson SR (1962) Medulloepithelioma of the retina. Int Ophthalmol occurred in 3 cases (8%) over a mean follow-up of 49 months, all of Clin 2: 483-506. whom presented with extra scleral extension of tumor due to mean 5. Mullaney J (1974) Primary malignant medulloepithelioma of the retinal delay in diagnosis by 39 months [6]. Distant metastases to the lymph stalk. Am J Ophthalmol 77: 499-504. nodes, parotid glands, lungs and mediastinum have been described. 6. Saunders T, Margo CE (2012) Intraocular medulloepithelioma. Arch Follow-up on the 56 patients reported by Broughton and Zimmerman Pathol Lab Med. 136: 212–216. showed tumour-related deaths in 4 (12%) occurred in